Abstracts tagged "interferon"

Abstract Number: 1949 • ACR Convergence 2020
CB2 Receptor Distribution and Effects of LenabasumTM in Dermatomyositis In Vitro
Spandana Maddukuri¹, Jay Patel², Christina Bax³, Maria Wysocka³ and Victoria Werth⁴, ¹University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Montville, NJ, ²University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Philadelphia, ³University of Pennsylvania, Department of Dermatology, Philadelphia, ⁴University of Pennsylvania and Corporal Michael J. Crescenz Veterans Administration Hospital, Philadelphia
Background/Purpose: Dermatomyositis (DM) patients report poor quality of life due to disease activity and persistent itch. Lenabasum is an oral non-immunosuppressive, non-psychoactive cannabinoid type 2…
Abstract Number: 0284 • ACR Convergence 2020
Oxidative DNA Damage Accelerates Skin Inflammation in Pristane-induced Lupus Model
Gantsetseg Tumurkhuu¹, Shuang Chen², Erica Montano¹, Malcolm Lane², Michifumi Yamashita², Janet Markman², Luz Blanco³, Mariana Kaplan⁴, Kenichi Shimada², Timothy Crother², Mariko Ishimori², Daniel J Wallace¹, Caroline Jefferies⁵ and Moshe Arditi², ¹Cedars-Sinai Medical Center, Los Angeles, ²Cedars-Sinai Medical Center, Los Angeles, CA, ³National Institute of Arthritis and Musculoskeletal and Skin Diseases, Centreville, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ⁵Cedars-Sinai Medical Center, West Hollywood, CA
Background/Purpose: Systemic Lupus Erythematosus (SLE) is a chronic inflammatory autoimmune disease in which type I interferons (IFN) play a key role. The IFN response can…
Abstract Number: 1067 • ACR Convergence 2020
Anti-Viral Proinflammatory Phenotype in Circulating Monocytes from Patients with Anti-Melanoma Differentiation-Associated Gene 5 Antibody-Associated Interstitial Lung Disease
Takahisa Gono¹, Yuka Okazaki¹ and Masataka Kuwana², ¹Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, ²Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Tokyo, Japan
Background/Purpose: Anti-melanoma differentiation-associated gene 5 (MDA5) antibody is associated with interstitial lung disease (ILD), which often represents rapidly progressive course and fatal outcomes. Circulating levels…
Abstract Number: 0286 • ACR Convergence 2020
An SLE-linked ITGAM Gene Variant Changes Mac-1 Structure, Signaling, and Surface Expression and Enhances IFNg Production and Antigen Presentation by B Cells
Joseph Blake¹, Alexander Szalai², Jeffrey Edberg³ and James Mobley³, ¹UAB, Birmingham, ²University of Alabama at Birmingham, birmingham, AL, ³UAB, Birmingham, AL
Background/Purpose: SLE is a chronic and debilitating disease; in the USA with an estimated incidence of 3-10 per 100,000 people and currently affecting an estimated…
Abstract Number: 1150 • ACR Convergence 2020
Traditional Laboratory Parameters and New Biomarkers in Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis
Arianna De Matteis¹, Denise Pires Marafon¹, Ivan Caiello¹, Manuela Pardeo¹, Giulia Marucci¹, Emanuela Sacco¹, Giusi Prencipe¹, Fabrizio De Benedetti² and Claudia Bracaglia¹, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ²Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy
Background/Purpose: Macrophage Activation Syndrome (MAS) and secondary Hemophagocytic Lymphohistiocytosis (sHLH) are hyperinflammatory conditions caused by a cytokine storm, in which IFNγ plays a pivotal role.…
Abstract Number: 0287 • ACR Convergence 2020
RNA Externalized by Neutrophil Extracellular Traps Promotes Inflammatory Pathways in Endothelial Cells
Xinghao Wang¹, Philip Carlucci², Jorge Romo-Tena¹, Jose Torres-Ruiz¹, Hong-Wei Sun¹, Markus Hafner¹, Mariana Kaplan³ and Luz Blanco⁴, ¹NIAMS, National Institute of Health, Bethesda, ²New York University School of Medicine, New York, NY, ³National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases, Centreville
Background/Purpose: Neutrophil extracellular traps (NETs) are extracellular lattices composed of nucleic material bound to neutrophil granule proteins. NETs may play pathogenic roles in development and…
Abstract Number: 1158 • ACR Convergence 2020
Clinical Features and Outcomes in STING-Associated Vasculopathy with Onset in Infancy (SAVI)
Sofia Torreggiani¹, Sara Alehashemi², Jacob Mitchell¹, Gema Souto Adeva¹, Bin Lin¹, Jenna Wade¹, Gina Montealegre Sanchez³, Abdulrahman Alrasheed⁴, Sibel Balci⁵, Roberta Berard⁶, Borzutzky Arturo⁷, Jürgen Brunner⁸, Bjoern Buehring⁹, Al Adba Buthaina¹⁰, Caterina Cancrini¹¹, John Carter¹², Mireia Corbeto Lopez¹³, Fabrizio De Benedetti¹⁴, Huy Do¹⁵, Gregor Dueckers¹⁶, Les Folio¹⁵, Antonella Insalaco¹⁷, Rabia Miray Kisla Ekinci⁵, Michael Miller¹⁸, Marco Montes Cano¹⁹, Marie-Paule Morin²⁰, Seza Ozen²¹, Lucia Pacillo¹¹, Suzanne Ramsey²², Adam Reinhardt²³, Dax Rumsey²⁴, Laisa Santiago²⁵, Grant Schulert²⁶, Benjamin Wright²⁷, Adriana de Jesus²⁸ and Raphaela Goldbach-Mansky²⁹, ¹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Clarksville, MD, ³NIAID/NIH, Rockville, MD, ⁴King Abdullah Specialized Children Hospital, Riyadh, Riyadh, Saudi Arabia, ⁵Department of Pediatric Rheumatology, Cukurova University Faculty of Medicine, Adana, Turkey, ⁶London Health Sciences Centre, London, ON, Canada, ⁷Pontificia Universidad Católica de Chile, Santiago, Chile, ⁸Tirol Kliniken, Innsbruck, Innsbruck, Austria, ⁹Rheumazentrum Ruhrgebiet, Ruhr-University-Bochum, Herne, Germany, ¹⁰Sidra Medicine, Doha, Doha, Qatar, ¹¹Unit of Immune and Infectious Diseases, Scientific Institute for Research and Healthcare (IRCCS) Childrens’ Hospital Bambino Gesù, University Department of Pediatrics (DPUO); Department of Systems Medicine, University of Rome Tor Vergata, Roma, Italy, ¹²University of South Florida, Tampa, FL, ¹³Vall d’Hebron Hospital Universitari, Vall d’Hebron Barcelona Hospital Campus, Barcelona, Spain, ¹⁴Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ¹⁵Radiology and Imaging Sciences, Clinical Center, NIH, Bethesda, ¹⁶Helios Kliniken - Kinderklinik, HELIOS Klinikum Krefeld, Germany, Krefeld, Germany, ¹⁷Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ¹⁸Feinberg School of Medicine, Northwestern University Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, ¹⁹Hospital Universitario Virgen del Rocío, Sevilla, Sevilla, Spain, ²⁰Université de Montréal, CHU Sainte-Justine, Montréal, Canada, ²¹Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Ankara, Turkey, ²²IWK Health Centre, Dalhousie University, Halifax, NS, Canada, ²³Boys Town National Research Hospital, Omaha, Omaha, NE, ²⁴Alberta Health Services – Edmonton Zone (Stollery Children’s Hospital), University of Alberta, Edmonton, AB, Canada, ²⁵Johns Hopkins All Children's Hospital, St. Petersburg, FL, ²⁶PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ²⁷Mayo Clinic, Phoenix, AZ, ²⁸Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD, ²⁹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD
Background/Purpose: STING-Associated Vasculopathy with Onset in Infancy (SAVI) is an autoinflammatory interferonopathy caused by gain-of-function mutations in STING1, characterized by peripheral vasculopathy and interstitial lung…
Abstract Number: 0289 • ACR Convergence 2020
Endogenous Interferon-β and Low IL-4R on Transitional B Cells Promotes Lupus Nephritis
Fatima Alduraibi¹, Huma Fatima¹, W. Winn Chatham¹, Hui-Chen Hsu¹ and John Mountz², ¹University of Alabama at Birmingham, Birmingham, AL, ²University Alabama at Birmingham and Birmingham VA Medical Center, Birmingham, AL
Background/Purpose: We previously showed that B-cell endogenous interferon-beta (IFNβ) at the transitional (Tr) stage correlates with development of anti-Smith (anti-Sm) and renal disease as well…
Abstract Number: 1159 • ACR Convergence 2020
Novel STING1 Mutations Including in the Transmembrane Linker Region Cause STING-associated Vasculopathy with Onset in Infancy (SAVI)
Bin Lin¹, Dana Kahle¹, Adriana Almeida de Jesus¹, Sofia Torreggiani², Jacob Mitchell², Alexander Aue¹, Zheng Ji³, Tengchuan Jin³ and Raphaela Goldbach-Mansky⁴, ¹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ³University of Science and Technology of China, Hefei, China (People's Republic), ⁴Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD
Background/Purpose: STING-associated vasculopathy with onset in infancy (SAVI) is an autoinflammatory disease caused by gain-of-function (GOF) mutations in STING1/TMEM173 that encodes stimulator of interferon genes,…
Abstract Number: 0292 • ACR Convergence 2020
Exhausted pSTAT5-IFNα Signaling Pathways in SLE Patients Are Correlated with Age-associated B Cells and Disease Activity
Samantha Slight-Webb¹, Miles Smith², Kevin Thomas¹, Susan Macwana¹, Holden Maecker³, Paul Utz⁴, Judith James⁵ and Joel Guthridge¹, ¹Oklahoma Medical Research Foundation, Oklahoma City, OK, ²Oklahoma Medical Research Foundation, Oklahoma City, ³Institute for Immunity, Transplantation and Infection, Stanford University School of Medicine, Stanford, CA, ⁴Stanford University School of Medicine, Stanford, CA, ⁵Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation;Department of Pathology, University of Oklahoma Health Sciences Center;Department of Medicine, University of Oklahoma Health Sciences Center, Edmond, OK
Background/Purpose: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by periods of elevated and suppressed clinical symptoms. Specific cell subsets, such as CD11c+ age-associated…
Abstract Number: 1399 • ACR Convergence 2020
Differential Impacts of TNFa Inhibitors on the Expression of Th Cytokines
Ching-Huang Ho¹, Andrea Silva¹ and I-Cheng Ho², ¹Brigham and Women's Hospital, Boston, MA, ²Birgham and Women's Hospital, Boston, MA
Background/Purpose: Inhibition of TNFα has emerged as an effective therapeutic approach for many autoimmune/inflammatory diseases.While the efficacy and safety profile of the five FDA-approved TNFis…
Abstract Number: 009 • 2020 Pediatric Rheumatology Symposium
Emapalumab (Anti-Interferon-Gamma Monoclonal Antibody) in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (sJIA)
Fabrizio De Benedetti¹, Paul Brogan ², Claudia Bracaglia ¹, Manuela Pardeo ¹, Giulia Marucci ¹, Emanuela Sacco ¹, Despina Eleftheriou ³, Charalampia Papadopoulou ³, Alexei Grom ⁴, Pierre Quartier ⁵, Rayfel Schneider ⁶, Philippe Jacqmin ⁷, Rikke Frederiksen ⁸, Maria Ballabio ⁹ and Cristina de Min ⁸, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, ²UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ³UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Section Head Infection, Immunology, and Rheumatology, London, United Kingdom, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, ⁵Paris-Descartes University, IMAGINE Institute, RAISE reference centre, Pediatric Immuno-Hematology and Rheumatology Unit, Necker hospital, Assistance Publique-Hôpitaux de Paris, Paris, France, ⁶The Hospital for Sick Children, Toronto, Canada, ⁷MnS Modelling and Simulation, Dinant, Belgium, ⁸Swedish Orphan Biovitrum AG (Sobi), Basel, Basel-Stadt, Switzerland, ⁹Swedish Orphan Biovitrum AG (Sobi), Basel, Switzerland
Background/Purpose: MAS is a severe complication of rheumatic diseases and occurs most frequently in patients with sJIA. Data from animal models and from observational studies…
Abstract Number: 5 • 2019 ACR/ARP Annual Meeting
Interferon Kappa Promotes the Development of Psoriasis
Mehrnaz Gharaee-Kermani¹, Shannon Estadt ¹, Sonya Wolf-Fortune ¹, Johann Gudjonsson ¹ and J. Michelle Kahlenberg ¹, ¹University of Michigan, Ann Arbor, MI
Background/Purpose: Psoriasis is a one of the most common chronic inflammatory autoimmune skin diseases characterized by hyperproliferation and abnormal differentiation of keratinocytes and infiltration of…
Abstract Number: 1021 • 2019 ACR/ARP Annual Meeting
Anti-IFNAR Treatment Does Not Reverse Neuropsychiatric Disease in MRL/lpr Lupus Mice
Michelle Huang¹, Ariel Stock ², Elise Mike ², Roland Kolbeck ³ and Chaim Putterman ⁴, ¹Albert Einstein College of Medicine, Bronx, ²Albert Einstein College of Medicine, Bronx, NY, ³MedImmune, Gaithersburg, MD, ⁴Albert Einstein College of Medicine, New York, NY
Background/Purpose: Many systemic lupus erythematosus patients display a type I interferon (IFN) signature, and IFNα levels and gene signatures have been positively correlated with disease…
Abstract Number: 1059 • 2019 ACR/ARP Annual Meeting
BDCA2 Targeting of Human Plasmacytoid Dendritic Cells via CBS004 Reverts Dependent IFN Activation and Tissue Fibrosis in vitro and in vivo
Rebecca Ross¹, Clarissa Corinaldesi ², Gemma Migneco ³, Yasser El-Sherbiny ⁴, Steve Holmes ⁵, Jörg Distler ⁶, Clive McKimmie ⁷ and Francesco Del Galdo ⁸, ¹Univesristy of Leeds, Leeds, England, United Kingdom, ²University of Leeds, Leeds, England, United Kingdom, ³University of Leeds, Leeds, United Arab Emirates, ⁴University of Nottingham, Nottingham, United Kingdom, ⁵Capella Bioscience, london, United Kingdom, ⁶Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, ⁷University of Leeds, Leeds, United Kingdom, ⁸University of Leeds and LTHT NIHR Biomedical Research Centre, Leeds, United Kingdom
Background/Purpose: Human plasmacytoid dendritic cells (pDCs) have been implicated in the pathogenesis of Systemic Sclerosis (SSc) through their ability to infiltrate the skin and secrete…